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一名年轻成年男性的原发性肾白血病,作为T细胞急性淋巴细胞白血病的髓外表现。

Primary renal leukaemia in a young adult male as an extramedullary presentation of T cell acute lymphoblastic leukaemia.

作者信息

Jain Punit, Jain Poonam, Ohgami Robert S, Pawar Veena, Sehgal Kunal, Chaudhari Pradnya, Nikalji Ravindra, Singh Tejinder, Khandelwal Vipin, Khare Sanjay, Lokhande Vaishali, Haridas Ashwathy, Jessani Laxman, Khandelwal Kanika

机构信息

Haematology Oncology and Stem Cell Transplant Unit Apollo Hospitals Navi Mumbai India.

Helix Genetic and Pathology Laboratory Mumbai India.

出版信息

EJHaem. 2023 Nov 15;5(1):251-255. doi: 10.1002/jha2.820. eCollection 2024 Feb.

Abstract

Primary renal involvement by T lymphoblasts is rare among adults with T acute lymphoblastic leukaemia. We report a 28-year-old man presenting with acute renal failure due to infiltration by T lymphoblasts and his response to paediatric-inspired modified BFM-90 protocol. The patient achieved an initial complete remission (CR) but developed central nervous system relapse. He achieved CR2 with cranial irradiation and intrathecal chemotherapy. He underwent a haploidentical transplant in CR2 and remains in remission post-transplant day 330. An early kidney biopsy helped confirm the diagnosis. Such presentations remain responsive to modified BFM-90. An early allotransplant in CR2 remains the standard of care.

摘要

在成人T急性淋巴细胞白血病中,T淋巴母细胞原发性肾受累情况罕见。我们报告了一名28岁男性,因T淋巴母细胞浸润出现急性肾衰竭,以及他对借鉴儿童方案改良的BFM - 90方案的反应。该患者最初达到完全缓解(CR),但出现中枢神经系统复发。经颅脑照射和鞘内化疗后他达到第二次完全缓解(CR2)。他在CR2期接受了单倍体同基因移植,移植后330天仍处于缓解状态。早期肾活检有助于确诊。此类表现对改良的BFM - 90方案仍有反应。CR2期早期进行同种异体移植仍是标准治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1eeb/10887257/8d44c28f1c6f/JHA2-5-251-g002.jpg

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